The placental component and obstetric outcome in severe preeclampsia with and without HELLP syndrome

HELLP syndrome “HELLP is an acronym that refers to a syndrome characterized by Hemolysis with a microangiopathic blood smear, Elevated Liver enzymes, and a Low Platelet count “(Weiner et al,2016). “It probably represents a severe form of preeclampsia, but the relationship between the two disorders remains controversial. As many as 15 to 20 percent of patients with HELLP syndrome do not have antecedent hypertension or proteinuria. Both preeclampsia with severe features and HELLP syndrome may be associated with serious hepatic manifestations, including infarction, hemorrhage, and rupture “(Weiner et al,2016). HELLP develops in approximately 0.1 to 0.2 percent of pregnancies overall and in 10 to 20 percent of women with severe preeclampsia/eclampsia. A previous history of preeclampsia or HELLP is a risk factor for HELLP syndrome. A variety of genetic variants associated with an increased risk of HELLP syndrome have been reported but have no role in clinical management. In contrast to preeclampsia, nulliparity is also a risk factor for HELLP syndrome but half or more of affected patients are multiparous (Laney, 2019). HELLP syndrome is a form of severe preeclampsia, it likely originates from aberrant placental development and function (Laney, 2019). As an independent entity, it has been attributed to abnormal placentation, like preeclampsia, but with greater hepatic inflammation and greater activation of the coagulation system than in preeclampsia. In a case of severe early HELLP syndrome, treatment with eculizumab, a targeted inhibitor of complement protein C5,is associated with marked clinical improvement and complete normalization of lab parameters for 16 days .This intervention is based on the fact that preeclampsia/HELLP is a systemic inflammatory disorder and the complement cascade is a key mediator, and the observation that women with mutations in complement regulatory proteins appear to be at increased risk of severe preeclampsia( Kuzmin,2018). Symptoms of HELLP are variable with the most common symptoms being abdominal pain and tenderness in the midepigastrium, right upper quadrant, or below the sternum. Hypertension and proteinuria are present in approximately 85 percent of cases, but it is important to remember that either or both may be absent in women with otherwise severe HELLP syndrome(Kuzmin,2018).”Signs and symptoms typically develop between 28 and 36 weeks of gestation, but second trimester or postpartum onset is also common. Serious maternal morbidity may be present at initial presentation or develop shortly thereafter. This includes disseminated intravascular coagulation (DIC), abruptio placentae, acute renal failure, pulmonary edema, subcapsular or intraparenchymal liver hematoma, and retinal detachment “(LaMarca, Cunningham, Cornelius, & Amaral, 2015). Precise criteria for diagnosing HELLP are: . Microangiopathic hemolytic anemia with characteristic schistocytes (also called helmet cells) on blood smear, Other signs suggestive of hemolysis include an elevated indirect bilirubin level and a low serum haptoglobin concentration (≤25 mg/dL). . Platelet count ≤100,000 cells/microL. . Total bilirubin ≥1.2 mg/dL (20.52 micromol/L; hemolysis results in an increase in indirect bilirubin) . Serum AST >2 times upper limit of normal for local laboratory (usually >70 international units/L)” (ACOG,2017). Some investigators obtain alanine aminotransferase (ALT) levels instead of, or in addition to, AST levels. An advantage of the AST is that it is a single test that reflects both hepatocellular necrosis and red cell hemolysis (ACOG,2017). women who do not meet all the above laboratory abnormalities are considered to have partial HELLP syndrome. However, these patients may progress to complete expression of HELLP syndrome (Kuzmin,2018). Management Approach The initial steps in management are to stabilize the mother, assess the fetal condition, and decide whether prompt delivery is indicated. Pregnancies less than 34 weeks of gestation, such as with the case of our given patient, and those in which the mother is unstable, should be managed in consultation with a maternal-fetal specialist. Antihypertensive drugs are used to treat severe hypertension. Hypertension can usually be controlled with labetalol, hydralazine, or nifedipine or, in severe cases, with sodium nitroprusside. Magnesium sulfate is given intravenously to prevent convulsions, and for fetal/neonatal neuroprotection in pregnancies between 24 and 32 weeks of gestation. Pregnancies ≥23 and <34 weeks of gestation, when both the maternal and fetal status are reassuring, administration of corticosteroids before delivering pregnancy complicated by HELLP syndrome at less than 34 weeks of gestation might be safe but delivery should proceed before 48 hours because delivery beyond 48 hours , disease progression usually occurs, sometimes with rapid maternal deterioration(LaMarca, Cunningham, Cornelius, & Amaral, 2015). Antihypertensive medication is given to control severe hypertension, as needed, and magnesium sulfate is given before delivery for neuroprotection. It is very necessary to repeats the complete blood count and platelet count at 24 and 48 hours after administering steroids, and use these results when considering whether to administer red blood cell transfusions, whether epidural can be performed safely, and whether platelet transfusion is indicated (Turley,2017). Labor can be induced in women with favorable cervices or pregnancies at least 30 to 32 weeks of gestation. However, cesarean delivery is probably preferable in pregnancies less than 30 to 32 weeks of gestation if the cervix is unfavorable for induction, especially if there are signs of fetal compromise (growth restriction, abruptio placenta, oligohydramnios). Induction of these pregnancies, even with use of cervical ripening agents, generally has a high failure rate and is often prolonged, thereby potentially exposing the mother and fetus to a higher risk of complications from severe HELLP syndrome. The American College of Obstetricians and Gynecologists’ Task Force (2017) on hypertension in pregnancy opined that dexamethasone may be justified before 34 weeks of gestation to raise the maternal platelet count. HELLP syndrome is associated with a variety of maternal morbidities, which can rarely result in a fatal outcome. The risk of serious morbidity correlates with increasing severity of maternal symptoms and laboratory abnormalities (Laney, 2019). There is no evidence that any therapy prevents recurrent HELLP syndrome, HELLP syndrome is considered as a form of severe preeclampsia and prescription of low-dose aspirin in future pregnancies is needed to reduce the risk of preeclampsia. The outcome for mothers with HELLP syndrome is generally good, but serious complications such as abruptio placentae, acute renal failure, subcapsular liver hematoma, pulmonary edema, and retinal detachment may occur. Future pregnancies are at increased risk of HELLP, preeclampsia, and gestational hypertension. To minimize this risk, after delivery maintain an ideal weight, eat a variety of fruits and vegetables, exercise regularly, and do not smoke (Weiner et al,2016). References The American College of Obstetricians and Gynecologists (ACOG) Task Force on (2017). Intensive Care Unit issues in eclampsia and HELLP syndrome cited frome; www.ncbi.nlm.nih.gov › pmc › articles › PMC5613404 HELLP Syndrome: Symptoms, treatment, and prevention. (2016, March 29). Retrieved February 9, 2019, from http://americanpregnancy.org/pregnancy-complications/hellp-syndrome/ Kuzmin, V. (2018). Preeclampsia and hellp syndrome – Obstetric prognosis. Pregnancy Hypertension, 13. doi: 10.1016/j.preghy.2018.08.160 LaMarca, B., Cunningham, M., Cornelius, D., & Amaral, L. (2015). Preeclampsia: long-term consequences for vascular health. Vascular Health and Risk Management, 403. doi:10.2147/vhrm. s64798 Laney. (2019, January 3). About HELLP Syndrome. Retrieved February 9, 2019, from https://www.preeclampsia.org/health-information/hellp-syndrome Turley, S. M. (2017). Hematology and immunology – Blood and lymphatic systems. In Medical terminology [Vault E-book] (4th ed., pp. 260-314). Retrieved from https://www.vaultebooks.com/#/books/9780134318431/cfi/3!/4/4@0.00:3.57 Weiner, E., Schreiber, L., Grinstein, E., Feldstein, O., Rymer-Haskel, N., Bar, J., & Kovo, M. (2016). The placental component and obstetric outcome in severe preeclampsia with and without HELLP syndrome. Placenta, 47, 99-104. doi: 10.1016/j.placenta.2016.09.012 References (continued) PLEASE RESPOND

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